Using computed tomography (CT), we have been able to identify the combined occurrence of lipoma of the corpus callosum and dysraphic lesions associated with frontal bone defects in two cases. In one case, there was a true encephalocele, and in the other case a lipoma with cartilaginous tissue was present. Such combinations are very rare; thus, the impetus to report these cases. The disorganization of the central nervous system was extensive in the first infant and was associated with profound developmental delay, while the second infant had much less anatomical abnormality, and development to date is normal. Bony defects (on skull films), typical appearances of lipoma of the corpus callosum, and characteristics of the dysraphic lesions (on CT) were seen. Angiography was obtained in the infant with a true encephalocele.